Fibrous dysplasia involving the temporal bone: report of three new cases

Monostotic fibrous dysplasia, an unusual disease that can involve the temporal bone, will frequently cause an acquired stenosis of the external auditory canal. Three patients with this disorder who presented with occluded canals are described. Two individuals developed external canal cholesteatomas medial to the obstruction, one of whom eventually developed a postauricular abscess and infected draining sinus. The third patient presented with a restenosis nine months following canalplasty. The cases reported illustrate three surgical criteria necessary to manage these unusual cases successfully: removal of sufficient diseased bone to create a patulous canal; resurfacing denuded bony areas with thin split-thickness skin grafts to prevent soft tissue contractions; an adequate meatoplasty. Postoperatively, the reconstructed canals have remained patent and stable during follow-up periods ranging from one to four years. Clinical, radiographic, and pathologic features of fibrous dysplasia are discussed, the differential diagnosis is presented, and the relevant literature is reviewed.